Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Nov 27, 2021; 13(11): 1727-1742
Published online Nov 27, 2021. doi: 10.4254/wjh.v13.i11.1727
Cystic fibrosis associated liver disease in children
Joseph J Valamparampil, Girish L Gupte
Joseph J Valamparampil, Girish L Gupte, Liver Unit, Birmingham Children’s Hospital, Birmingham B4 6NH, United Kingdom
Author contributions: Gupte GL contributed to the conceptualization and intellectual input of final draft of manuscript; Valamparmpil JJ contributed to primary draft of manuscript, data collection and literature retrieval; and both authors contributed equally to the manuscript.
Conflict-of-interest statement: No conflict of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Girish L Gupte, MD, Consultant Physician-Scientist, Liver Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, United Kingdom.
Received: June 11, 2021
Peer-review started: June 11, 2021
First decision: July 6, 2021
Revised: July 19, 2021
Accepted: September 16, 2021
Article in press: September 16, 2021
Published online: November 27, 2021
Core Tip

Core Tip: Cystic fibrosis(CF) liver disease is caused by abnormal cholangiocyte function, altered biliary secretion and abnormal innate immune response with abnormal response to endotoxins. CF liver disease can present with a wide variety of clinical features from a heterogenous liver on ultrasound, to life threatening gastrointestinal bleeds secondary to portal hypertension. Novel treatment strategies directly targeting the ion channel abnormality-cystic fibrosis transmembrane conductance regulator modulators are available and has significantly improved the clinical status and life expectancy of the cystic fibrosis patients.